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ILD.pptx
- 1. Dr Nivedita Charkrabarty DrAayush Dr Swetha Interstitial lung disease
- 2. Definition • Heterogeneous groupof diffuse lung diseases occurring without known cause and associated with varying degrees of interstitial lung inflammation and fibrosis.
- 3. Classification ILDs Major Chronic Fibrosing 1.UIP 2. NSIP Smoking related 3. DIP 4. RB Acute/Subacute 5. Organising pneumonia 6. DAD Rare 7. Lymphoid IP 8. Pleuropulmonary fibroelastosis
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- 6. USUAL INTERSTITIAL PNEUMONIAAND IDIOPATHIC PULMONARY FIBROSIS • Most common, 40% • UIP: histologic pattern, IPF: disease • Heterogeneous pattern: "spatial" and "temporal“ heterogeneity • >50 yrs • progressive dyspnea, cough, weight loss, and finger clubbing • PFT: restrictive pattern
- 7. Clinical associations • Collagen-vasculardisease, • Asbestosis, • Drug toxicity, • Radiation, • Chronic hypersensitivity pneumonitis, or familial pulmonary fibrosis
- 8. • In earlystages, the only HRCT abnormality may be fine reticulation • Honey combing 3mm to 2 cm, 3 in a row • reticular opacities • traction bronchiectasis • subpleural and lower lobe predominance • GGOs are rare
- 10. Combined pulmonary fibrosisand emphysema Diagnostic Criteria ATS and ERS • 1. A UIP pattern on HRCT or histopathology • 2. The absence of an alternative etiology (e.g., collagen-vascular disease, asbestos exposure, drug treatment, hypersensitivity pneumonitis) after careful clinical evaluation (i.e., history, physical examination, pulmonary function testing, and laboratory assessment)
- 11. UIP pattern • 1.Subpleural and basal predominance • 2. Reticular abnormality • 3. Honeycombing with or without traction bronchiectasis • 4. Absence of inconsistent features
- 12. Possible UIP • 1.Subpleural and basal predominance • 2. Reticular abnormality • 3. Absence of inconsistent features
- 13. Inconsistent with UIP •1. Upper or midlung predominance • 2. Peribronchovascular predominance • 3. Extensive ground-glass abnormality (extent > reticulation) • 4. Profuse micronodules (bilateral, predominantly upper lobes) • 5. Discrete cysts (multiple, bilateral, away from areas of honeycombing) • 6. Diffuse mosaic attenuation/air trapping (bilateral in three or more lobes) • 7. Segmental or lobar consolidation
- 16. Radiography • CXR: subtle increasein opacity at lung base
- 17. NONSPECIFIC INTERSTITIAL PNEUMONIA •Less common • Specific pathological entity • Alveolar wall thickening or fibrosis on HPE • Spatial and temporal homogeneity • Cellular NSIP • Fibrotic NSIP
- 18. Presentation • Younger agethan UIP 40-50 yrs • 18 to 30 months symptom duration • Responds to steroids • Good prognosis
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- 20. • Ground-glass opacityor reticulation • “without“ honeycombing • subpleural and basal lung • subpleural sparing
- 22. ORGANIZING PNEUMONIA (CRYPTOGENICORGANIZING PNEUMONIA) • Patchy areas of organizing pneumonia • mononuclear cells, foamy macrophages, and organizing fibrosis • peripheral airspaces, including bronchioles, alveolar ducts, and alveoli • 10 to 20%, common • BOOP • Idiopathic OP: COP • A/w CVD (Myositis syndromes), pulmonary infection, drug reactions, bronchial obstruction, aspiration, and after toxic fume inhalation
- 23. Presentation • Several monthh/o non productive cough, low grade fever, malaise, dyspnea • ~55 years • Symptom duration shorter, more systemic symptoms • Steroid responsive • Good prognosis
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- 25. Patterns • Patchy consolidationor GGO (60%), subpleural and/or peribronchial distribution • Small, ill-defined nodules, peribronchial or peribronchiolar • Large nodules or masses, which may be irregular in shape • Focal or lobar consolidation • reversed halo sign or atoll sign m/c drug reactions • Perilobular pattern m/c with myositis syndromes • Overlapping features with CEP
- 26. Differentials of Atoll/Reversehalo sign • Infectious diseases: invasive aspergillosis, histoplasmosis, cryptococcosis, PCP, TB, Legionella • Non infectious: COP, Secondary organizing pneumonia • Vasculitis • Sarcoidosis • Lipoid pneumonia • Lymphomatoid granulomatosis
- 27. ACUTE INTERSTITIAL PNEUMONIA •Fulminant disease of unknown cause • Histologically DAD • ~50 yrs, prodrome viral like • mechanical ventilation within 2 weeks • Hamman Rich syndrome/ Idiopathic ARDS • Poor prognosis
- 29. DESQUAMATIVE PNEUMONIA, RESPIRATORYBRONCHIOLITIS, RESPIRATORY BRONCHIOLITIS-INTERSTITIAL LUNG DISEASE • Different degrees of lung involvement by the same process • RB mildest, DIP most severe • Alveolar macrophagic infiltrate: diffuse in DIP, peribronchiolar in RB • Strong association with smoking, with cessation prognosis is very good
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- 32. LYMPHOID INTERSTITIAL PNEUMONIA •histology: dense lymphoid interstitial infiltrate • Extensive alveolar wall involvement • Small airways with lymphoid follicles: Follicular bronchiolitis • A/w CVD, immune deficiency, autoimmune disorder, drug toxicity
- 33. Patterns • Patchy areasof GGO: immune deficiency • Centrilobular nodules, Ill defined: AIDS, CVD • Small, well-defined nodules perilymphatic distribution or septal thickening: AIDS • Isolated cystic airspaces: smog rents and other cvds
- 36. PLEUROPARENCHYMAL FIBROELASTOSIS • Upperlobe predominant elastotic fibrosis of pleura and adjacent parenchyma • Temporally homogeneous • Idiopathic or a/w nonspecific autoantibodies, familial, BMT, lung transplantation with restrictive allograft syndrome, or infection • Dry cough, SOB for several yrs • Progressive • predominantly upper lung zone pleural thickening • subpleural reticulation • upper lobe volume loss • upward retraction of the hila
- 38. LYMPHANGIOLEIOMYOMATOSIS • progressive proliferationof perivascular epithelioid cells (PECs) in relation to bronchioles, small pulmonary vessels, and lymphatics in the chest and abdomen • low-grade destructive metastasizing malignancy • Associated with Tuberous sclerosis • almost exclusively in women of childbearing age • dyspnea, pneumothorax, or cough • 60% chylous pleural effusions; 80% pneumothoraces; 30% to 40% blood- streaked sputum or frank hemoptysis
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- 42. Case Profile: 63year old male, presenting with progressive dyspnea, with new onset requirement of oxygen aid.
- 43. Case Profile: An33 year old female, known case of ALL, post BMT, presenting with new onset shortness of breath, fever, chills and cough.
- 44. Diagnosed with Invasiveaspergillosis, and started on Voriconazole.
- 45. Known case ofpulmonary tuberculosis, with increasing breathlessness, malaise and one episode of hemoptysis. Sign?
- 46. Air crescent sign/Monod Sign of fungal ball
- 47. Case Profile: Youngfemale patient of Hodgkin’s lymphoma on ABVD regimen, presenting with dyspnea, cough and occasional fever. Condition and likely causative agent?
- 48. • Diffuse alveolarDamage • Bleomycin
- 49.